Specifications

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Applications

SDS-PAGE

Endotoxin level

< 1.000 Eu/µg

Purity

>95% by SDS-PAGE .

Form

Lyophilised

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Preparation and Storage

Stability and Storage

Shipped at 4°C. Store at 4°C prior to reconstitution. Store at -80°C. Avoid freeze / thaw cycle. For long term storage it is recommended to add a carrier protein on reconstitution (0.1% HSA or BSA).

pH: 7.50Constituents: 0.61% Tris, 0.75% Glycine, 5% Trehalose

Reconstitution

It is recommended to reconstitute the lyophilized product in 200 µl sterile deionized water to a final concentration of 1 mg/ml. Solubilize for 30 to 60 minutes at room temperature with occasional gentle mixing. Carrier protein (0.1% HSA or BSA) is strongly recommended for further dilution and long term storage.

Defects in IFNGR1 are a cause of mendelian susceptibility to mycobacterial disease (MSMD) [MIM:209950]; also known as familial disseminated atypical mycobacterial infection. This rare condition confers predisposition to illness caused by moderately virulent mycobacterial species, such as Bacillus Calmette-Guerin (BCG) vaccine and environmental non-tuberculous mycobacteria, and by the more virulent Mycobacterium tuberculosis. Other microorganisms rarely cause severe clinical disease in individuals with susceptibility to mycobacterial infections, with the exception of Salmonella which infects less than 50% of these individuals. The pathogenic mechanism underlying MSMD is the impairment of interferon-gamma mediated immunity whose severity determines the clinical outcome. Some patients die of overwhelming mycobacterial disease with lepromatous-like lesions in early childhood, whereas others develop, later in life, disseminated but curable infections with tuberculoid granulomas. MSMD is a genetically heterogeneous disease with autosomal recessive, autosomal dominant or X-linked inheritance.